About the Registry
This registry was created by the Pulmonary Fibrosis Foundation (PFF) back in 2016 after the organization sought input from PF researchers on their greatest unmet needs. It was clearly evident that the absence of a registry was inhibiting researchers from taking critical steps they needed. The PFF registry was formed. It is a national registry, including patient data collected from clinics across the country. These clinics are all participating members of PFF’s Care Center Network, or CCN. This ensures standard data collection, all of which is de-identified.
The registry currently contains patient data from 42 different CCN sites. In total, 2,002 Pulmonary Fibrosis (PF) and Idiopathic Pulmonary Fibrosis (IPF) patients are enrolled. However, with more funding, the PFF hopes to increase this enrollment to 6,000 patients by the year 2020.
Even at 2,000 patients, this registry is currently the largest PF database of its kind. It has, so far, aided 23 different research studies. Collectively, these investigations have received more than 30 million dollars in funding.
The data included in this registry supports researchers uncovering new causes of the disease, discovering new treatment options, working to improve the way we diagnosis the disease, working to improve disease management, and tracking the effects of current treatments.
The registry includes the following patient information-
- How their diagnosis was made
- Test results
- Medications used
- Blood sample (if the patient chooses)
- Data on how the patient is doing in the years following enrollment
In the future, the PFF hopes to expand their registry to-
- Include more forms of data (ex. data from pulmonologists)
- Include greater numbers of patient blood samples
- Include more patient data from individuals who have less-studied forms of the disease
- Share the registry data with more scientists to help increase understanding and research of PF
- Use the data to reassess and work to improve clinical guidelines
The ultimate goal of this registry is to accelerate the path to a cure for Pulmonary Fibrosis.
2019 ATS Presentations
The 2019 ATS Conference was held in Dallas, Texas May 17th-22nd. Some of the studies showcased at this conference were made possible due to the PFF registry data.
Below is a quick summary of these studies, and links to their full abstracts.
- There is a strong correlation between patient’s cough and the severity of their lung function impairment. Read the full abstract here.
- There are four distinct subgroups of PF patients with differing demographic, lung function, serological, and clinical data. Read the full abstract here.
- African-Americans typically receive their PF diagnosis a decade before those of European-American descent however there is no difference between mortality rates of these groups. Read the full abstract here.
- PF patients taking anticoagulants have a higher mortality rate than those not taking this medication. This risk was higher still in those taking anticoagulant warfarin as opposed to direct-acting oral anticoagulants. Read the full abstract here.
You can read more about these studies and other ways that the PFF registry is utilized here.